Surgical treatment of Trigeminal Neuralgia by MVD surgery is a fairly straightforward one; but if it is complicated by a cerebellar AVM, there are many twists and turns that could potentially be serious.
A rare and technically challenging Brain surgery performed by Dr Jaydev Panchawagh and his team gives a new lease of pain-free life to African woman at Pune.
Arterio-Venous-Malformations (AVM)
AVMs occurring inside the brain are formidable Neurosurgical challenges.
What is an AVM?
An AVM is an area of a densely packed conglomerate of malformed blood vessels. Normally an artery supplying a particular part of brain divides into smaller ‘arterioles’ and then even smaller ‘capillaries’. These smaller vessels have a strong muscular coat in their walls so that they can shrink in diameter to allow the blood flow in brain tissue to be slow and steady for the oxygen delivery to happen effectively.
The used blood is then collected by draining veins which open into bigger venous channels to take it to lungs eventually.
Why is an AVM dangerous?
In an AVM, not only are the vessels malformed, but the muscular layer in the smaller capillaries is absent, making them unable to contract. Also the smaller capillary layer is often absent. This causes direct shunting of arterial blood to the veins at very high speeds and flow rates.
AVMs in the brain are even more formidable as they occur in very important areas and their removal is considered one of the most difficult of brain surgeries…..which are , as a group challenging in the first place.
Secondly, brain being the most prized organ for the body, it’s blood flow follows slightly different rules than the remainder of the body. “Blood brain barrier” and “Cerebral blood flow Autoregulation” are only two examples of its (brain’s) unique nature.
Some of these unique characteristics could also be absent in AVMs, making them difficult to be tamed by surgery.
Another problem with the AVM is a “steal” phenomenon. As the name suggests, due to a very high flow across the AVM and reduced resistance for the flow in this area, some of the blood supply of the surrounding brain is “stolen” by the AVM.
The blood vessels which lead to the AVM, though not directly taking a part in formation of the AVM, increase in diameter, become tortuous and thicken due to continuous higher blood stream flowing through them.
Is AVM dangerous?
Brain AVMs can present with sudden bleeding or fits.
AVMs presenting with compression of the nerves causing neuralgia though known is extremely rare.
Details of this rare case….intolerable Face pain
In the rarest of rare, and surgically challenging case, a 54 year old eastern African patient was treated by combination therapy of Angiography led Embolization followed by Brain surgery to remove the malformation completely.
She was suffering from severe pain in the distribution of Trigeminal nerve and Glossopharyngeal Nerve for last many years.
The pain became intolerable even on very high doses of medicines.
Trigeminal Neuralgia and Glossopharyngeal Neuralgia in same patient
For her double Neuralgia, (Trigeminal Neuralgia and Glossopharyngeal Neuralgia) she came to the MVD surgery Centre at Pune.
But in her case, a simple MVD surgery would not have been sufficient as a more formidable problem in the form of Cerebellar AVM was present in the same area as the nerves.
In fact, the nerves were getting compressed due to high engorged blood vessels supplying the AVM.
Thus it was a much complex problem.
The treatment was planned with a detailed planning sessions with Neurosurgeon Dr Jaydev Panchwagh and interventional vascular specialist Dr Alurkar.
“This was rarest of the rare case, as we had to not only relieve her of extreme pain but while doing it, needed to eliminate the AVM completely.”
Staged operation
In the first stage, AVM was partly occluded by Angiography guided intervention technique.
In this a very thin catheter is inserted in the blood vessels supplying the AVM and medical glue is injected into the vessels.
This glue flows forward a bit and immediately becomes hard, this occluding the AVM blood vessels.
“However, we have to be careful as, if even a small excess amount is injected, it could occlude normal blood vessels or it can flow in normal circulation causing obstruction at undesired sites”
“Also due to tortuosity and curvaceous nature of AVM vessels, some of the vessels may not be approachable for threading the catheter.”
Therefore, after safe embolization, the AVM needs to be removed to ensure instant cessation of bleeding risk.”
“AVM surgery needs skilled operator and has to be done with meticulous planning and with the most modern Neurosurgical equipment.
After embolization, this AVM was still getting blood supply from multiple arteries and was in the cerebellum.
During the surgery, we have to be very careful to separate very important blood vessels which are “passing'” next to the AVM but not supplying it. They are the “transiting vessels” they need to be preserved.
Also, the actual feeding vessels are high flow vessels and need secure clipping before cutting them.
After 8 hour long meticulous surgery, we had to do the most important part.
That was to separate the blood vessels compressing the trigeminal and Glossopharyngeal nerves to treat her pain of Trigeminal and Glossopharyngeal double neuralgia.”
What is MVD surgery?
MVD surgery is a brain surgery during which the affected nerve..in this case the Trigeminal nerve and glossopharyngeal nerve is made free of compression from the offending blood vessel by using a piece of teflon pad. In experienced hands it has low risk.
Trigeminal neuralgia medication, at least the majority of them, are anti-convulsants.
“How to take them-Trigeminal neuralgia medication?”
Can I take TN medication when I feel the pain coming on?
Can I add more drugs during a Trigeminal neuralgia attack?
Do I have to continue taking the Trigeminal neuralgia medicines even if I have no or minimal pain?
These are all questions that frequently come to the patients’ minds.
What is essential to understand is that TN medicines are not straight-forward pain-killers. They are primarily anti-convulsants or brain numbing medications. Once the first pill is consumed, it takes a few hours to reach the blood. The liver simultaneously metabolizes the drug, while some of it also reaches the brain to act upon. So in effect, the blood level reduces. Over a period of the next few hours, the blood level will become zero. Before this happens, we need to consume another pill to increase and maintain the blood level. If this does not happen, there is no effect on the pain as well.
In short, regular medication is essential to maintain a particular blood level of the drug, so that it calms the brain/ nerve. This calming effect means that they do not respond to pain signals, and therefore have a neuralgic-pain-reducing effect.
Another key point to be noted is that the dosages need to be very gradually increased (and decreased also).
What happens if I take too many TN pills?
Well, you will certainly start manifesting all the side-effects of these anti-convulsants, like drowsiness, dizziness, imbalance, mental clouding, memory loss, depression etc. This is why, their dosages have to be adjusted by your doctor, who will determine what is right for you. One typically starts with the lowest dosage twice or thrice a day. This may be increased to maximum doses four times a day.
What happens if I miss a couple of doses of TN medication?
The blood level reduces, and you may experience break-through pain.
Allergic reactions to Trigeminal neuralgia medication
When starting off drugs to control TN pain for the first time, one has to be aware of and be on the lookout for allergic reactions. Though rare, it is not unheard of. I have come across a few patients in my practice. Typically they are in the form of skin rashes. These may be the harbinger of a very serious and potentially fatal Stevens-Johnson syndrome. If one notices skin rashes on consuming TN medication, please inform your doctor immediately and stop taking it.
For those of you who are wondering whether Trigeminal neuralgia is hereditary, the short answer is ‘ not really’. Having said that, there are cases reported where TN runs in families.
Can Trigeminal neuralgia be Familial?
Familial TN is unusual by all means. It is likely to be less than 2 or 3% of all TN patients. It is possible that patients inherit their familial ‘make-up’ of blood vessel contours or laxity, or lengths…we don’t know. Generally the familial type of Trigeminal neuralgia is on the same side in all affected family members.
Can Trigeminal neuralgia be inherited? Can TN be genetically transmitted?
The chances are so slim, that it’s not worth losing good sleep over it.
Have I seen a case of familial Trigeminal neuralgia?
‘No. I have only read case reports. It is likely that this condition is so rare, that one need not be too worried about it.’ Dr Jaydev Panchwagh answers.
Can children be affected with Trigeminal neuralgia?
Though it is unusual, it is not impossible. Diagnosing TN in children may be a problem, because they may not be able to express their problem clearly.
Parents ought to be more observant and help in establishing patterns.
My father and I both have Trigeminal Neuralgia. Is this hereditary? Will my children also have it?
It is possible that there are certain anatomical variations in either the brain and skull, or in the blood vessels that have predisposed both of you to have it. We just have to wait and see if it gets passed on to the next generation.
The youngest patient Dr Jaydev Panchwagh, a renowned Indian neurosurgeon has operated on is a girl of 22 years, who had been having it for five years. The operation was performed at his centre for MVD in Pune, India. With an experience of more than a thousand trigeminal neuralgia surgeries, he is one of the best neurosurgeons for such delicate and specialized operations.
You may watch his Youtube channel for more on MVD surgery for Trigeminal neuralgia treatment.